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Objective: To analyze the characteristics of septic pulmonary embolism (SPE) in intravenous drug users., Methods: The clinical manifestations, radiographic findings, bacteriology, echocardiography and outcome of intravenous drug users were analyzed retrospectively., Results: Twenty-two patients were identified with SPE between January of 1994 and December of 2006. Presenting symptoms included fever (22/22), dyspnea (20/22), pleuritic chest pain (10/22), cough (18/22), and hemoptysis (8/22). Chest radiographic features included nodular (15/22) and focal (12/22) infiltrates, wedge-shaped lesions (5/22), cysts (18/22), cavities (11/22), and pleural lesions (11/22). Peripheral or subpleural zones were most commonly affected (20/22). CT was more helpful and revealed multiple air cysts or nodules peripherally, often with cavitation. Staphylococcus aureus was the aetiological pathogen in all patients. Tricuspid valve vegetations were detected in all patients. Aside from antimicrobial therapy, the management included mechanical ventilation, control of shock and tube thoracostomy. Most patients recovered from their illness., Conclusions: SPE manifests with variable and often nonspecific clinical and radiographic features. The diagnosis is usually suggested by the presence of a predisposing factor, fever, and radiographic findings of multiple, peripheral or subpleural air cysts, or nodules with or without caritation. With early diagnosis, appropriate antimicrobial therapy, and control of the infectious source, resolution of the illness can be expected for most patients.

Background: Mucosa-associated lymphoid tissue (MALT) lymphoma of the larynx is a rare but well-documented entity which may arise from chronic inflammatory process. Supraglottic left regions are predominant due to unknown reason., Case Report: We present the case of a 62-year-old man with a dry cough, stridor and developing exertional dyspnea. This subglottic almost circumferential MALT lymphoma showed a temporary distinct disappearance after cortisone administration during the diagnostic process. Bronchoscopy confirmed the diagnosis of a primary MALT lymphoma of the larynx. The patient received chemotherapy according to CHOP scheme plus rituximab. A reliable post-treatment care period of 15 months showed no sign of recurrence., Conclusion: MALT lymphoma of the larynx are believed to arise from preexisting or acquired lymphoid tissue of the upper airway. Acquired lymphoid tissue is documented in the supraglottic region and may be associated with a chronic inflammatory process. However, in subglottic cases it is unclear whether the chronic inflammation arises from a local or systemic process.

Magnetic resonance (MR) and computed tomography (CT) were performed in an 8-year-old, spayed female cat with chronic effort respiration at the inspiration phase and stertor. Increased bone opacity in the areas of the head, neck and thorax were observed on radiography. MR images showed no signal intensity on both transverse T1WI and T2WI of the nasal cavity. CT revealed increased bone density and hypertrophy of the nasal turbinate and a narrowed nasal passage. From these results, we concluded this case had osteopetrosis-like disease, and that the respiratory distress was caused by hypertrophy of the nasal turbinate.

Fabry disease affects multiple organs, including the lungs. To date, there have been no reports of the effect of enzyme replacement therapy on patients with pulmonary manifestations of the disease. We report on a case of marked improvement of pulmonary signs and symptoms in a patient with Fabry disease following enzyme replacement therapy. A 38-year-old female with Fabry disease presented with dyspnea requiring constant supplemental oxygen. Following bi-weekly treatment with agalsidase beta, she demonstrated marked improvement in her pulmonary symptoms, eventually no longer needing supplemental oxygen and returning to an active lifestyle. Improvement was seen on pulmonary function tests and chest CT examinations. Enzyme replacement therapy may alleviate pulmonary dysfunction in patients with Fabry disease., ((c) 2007 Wiley-Liss, Inc.)

An 82-year-old female patient with a history of deep vein thrombosis presented with progressive dyspnea. Echocardiogram demonstrated significant pulmonary hypertension and patent ductus arteriosus (PDA). There was considerable debate regarding the role of PDA in the patient's pulmonary hypertension. The patient died of heart failure a few months later. Autopsy demonstrated extensive chronic pulmonary thromboembolic disease. Pulmonary thromboemboli continue to be a diagnostic challenge despite modern diagnostic modalities. Autopsy continues to play a role in investigating unexplained clinical findings and in determining cause of death.

Study Objective: The present study evaluated the relationship between health status (HS) and peripheral muscle histochemical characteristics in chronic obstructive pulmonary disease (COPD), and identified selected independent respiratory and extrapulmonary variables that predicted the HS of these patients., Design: Cross-sectional study., Setting: Outpatient respiratory clinic of a university hospital., Patients and Methods: We studied 29 patients (63+/-10 yrs) with a forced expiratory volume in 1s (FEV1) of 39+/-12%. All patients underwent vastus lateralis muscle biopsies for histochemical analysis. They also had spirometry, arterial blood gas analysis, body mass index (BMI), dyspnea determined with the MMRC scale and responded to the St. George's Respiratory Questionnaire (SGRQ) for HS assessment., Results: SGRQ total score correlated with fiber type distribution. A stepwise multiple regression identified three independent predictors of SGRQ total score: type I fiber proportion, BMI, and FEV1; r = 0.78 and r2 = 0.61., Conclusions: These results indicate that impaired HS in COPD is related to the peripheral muscle changes characterized by less type I fibers proportion. The findings argue in favor of an important contribution of the systemic consequences on the HS in COPD independently from the airflow limitation severity, and help to explain the observation of the poor correlation between the degree of airflow limitation and SGRQ total score.

There is a substantial need for a diagnostic tool to aid in the early diagnosis of heart failure and in the recognition of those at risk for its development, as well as in guidance of therapy. Testing for amino-terminal pro-brain natriuretic peptide (NT-proBNP) has been recognized to have utility in the diagnosis, prognosis and management of heart failure. In addition, numerous other applications for NT-proBNP testing are now recognized, such as evaluation of patients with heart disease in the absence of heart failure, as well as the diagnostic and prognostic evaluation of patients with acute coronary syndromes or pulmonary thromboembolism.

Background Context: Kyphoscoliosis is one of the most frequent complications of osteomalacia, which only rarely results in severe deformity requiring surgery. To the best of our knowledge, there has been only one previous report of a spinal deformity as a complication of osteomalacia that was sufficiently severe so as to require surgical treatment., Purpose: To report here the case of a 27-year-old woman who experienced back pain of gradual onset accompanied by progressive scoliosis resulting in severe dyspnea., Study Design: A case report., Methods: She was diagnosed with hypophosphatemic osteomalacia and secondary hyperparathyroidism. She underwent posterior surgical correction and fusion from Th4-L1 using the ISOLA spinal system., Results: At the last follow-up (3 year and 9 months postoperatively), her body balance was good and the dyspnea had disappeared. Plain radiographs demonstrated no loss of correction and also showed no evidence of instrumentation failure., Conclusions: We present a unique instance of a young woman with severe kyphoscoliosis who underwent posterior surgical correction/fusion with spinal instrumentation.

Context: D-dimer concentration can be used to exclude a diagnosis of acute pulmonary embolism. However, clinicians frequently order unnecessary supplemental testing in patients with low concentrations of D-dimer. Elevations in natriuretic peptides have also been described in the setting of pulmonary embolism., Objective: We investigated the integrative role of D-dimer with amino-terminal pro-B-type natriuretic peptide for the evaluation of patients with and without acute pulmonary embolism., Design: Patients were selected for analysis from a previous study in which levels of D-dimer and amino-terminal pro-B-type natriuretic peptide were measured. The presence of pulmonary embolism was determined by computed tomographic angiography., Results: The median levels of D-dimer were significantly higher in patients with acute pulmonary embolism. Similarly, the median levels of amino-terminal pro-B-type natriuretic peptide were higher in patients with pulmonary embolism., Conclusions: The Roche Tina-quant D-Dimer immunoturbidimetric assay provides a high negative predictive value and can be used to exclude acute pulmonary embolism in patients with dyspnea. Measurement of amino-terminal pro-B-type natriuretic peptide in addition to D-dimer improves specificity for acute pulmonary embolism without sacrificing negative predictive value. A combination of both markers may offer reassurance for excluding acute pulmonary embolism, and thus avoid redundant, expensive confirmatory tests.

Background: The aim of the present study was to demonstrate the capability of B-type natriuretic peptide (BNP) and amino terminal proBNP (NT-proBNP) as prognostic markers in patients with dyspnoea as a chief complaint., Methods: BNP and NT-proBNP plasma concentrations were obtained from 251 short of breath patients presenting to the emergency department of a tertiary care hospital. Patients with acute coronary syndromes or trauma were excluded. The endpoint was defined as all-cause mortality, and the study participants were followed up for 365 days from the time they attended the emergency department., Results: Of the 251 patients, 62 died and 189 stayed alive during follow-up. In the present study, optimal cut off levels for the prediction of survival were 454 ng/L for BNP, and 2060 ng/L for NT-proBNP. Mortality was higher in patients with baseline BNP and NT-proBNP concentrations above these cut off levels (log rank p<0.001; hazard ratios, 0.325 and 0.357, respectively). In multivariate Cox proportional-hazards regression analyses, elevated BNP/NT-proBNP, low systolic blood pressure, and renal dysfunction were predictors of mortality even when the baseline diagnosis of acute destabilized heart failure was factored into the model., Conclusions: Both BNP and NT-proBNP measures obtained from short of breath patients presenting to an emergency department may be predictive of one-year all-cause mortality independently of the baseline diagnosis of acute destabilized heart failure.

Although chronic obstructive pulmonary disease (COPD) patients frequently report symptoms, it is not known which factors determine the course of symptoms over time and if these differ according to the sex of the patient. The current study investigated predictors for presence, development and remission of COPD symptoms in 816 males and 312 females completing 3-yr-follow-up in the European Respiratory Society Study on Chronic Obstructive Pulmonary Disease (EUROSCOP). The following were included in generalised estimating equations logistic regression analyses: explanatory variables of treatment; pack-yrs smoking; age, forced expiratory volume in one second % predicted (FEV1 % pred); annual increase in FEV1 and number of cigarettes smoked; body mass index; and phadiatop. Interaction terms of sex multiplied by explanatory variables were tested. Over 3 yrs, similar proportions of males and females reported symptoms. In males only, higher FEV1 % pred was associated with reduction in new symptoms of wheeze and dyspnoea, and symptom prevalence was reduced with annual FEV1 improvement and phlegm prevalence reduced with budesonide treatment (odds ratio 0.66; 95% confidence interval 0.52-0.83). Additionally an increase in the number of cigarettes smoked between visits increased the risk of developing phlegm (1.40 (1.14-1.70)) and wheeze (1.24 (1.03-1.51)) in males but not females. The current study shows longitudinally that symptom reporting is similar by sex. The clinical course of chronic obstructive pulmonary disease can differ by sex, as males show greater response to cigarette exposure and treatment.

Objective: A clinical staging system for children with lymphatic malformations that would allow for a standardized comparison of disease and treatment outcomes., Methods: We developed an examination sheet for the determination of a disease score ranging from 0 (worst) points to 10 (best) points, Cologne Disease Score (CDS). Disfigurement, dysphagia, dysphonia, dyspnea and an observer statement towards progression were contributing to CDS. Each parameter yielded two, one or zero points. Two points were given when no limitation was seen in the patient concerning the respective item. One point was given at mild limitation and zero points were given when considerable limitation in the respective item could be observed. We evaluated 26 patients with lymphatic malformations of the head and neck retrospectively by completing one examination sheet for each patient-visit., Results: Four patients had an initial CDS value of three or less points (severe disease-group). After therapy the mean value of their score increased only slightly to 3.9+/-2.6 points. Six patients had an initial CDS value of four, five or six points (advanced disease-group). After treatment their mean score value increased dramatically to 9.0+/-1.5 points (P<0.001). Sixteen patients initially had seven points or higher (moderate disease-group), they had 9.8+/-0.4 points after treatment., Conclusions: The evaluation of the CDS was easy and expeditious. The score itself was a good predictor in view of the outcome. This new disease score for paediatric patients might be appropriate to evaluate therapeutic trials in paediatric patients.

Aim: To evaluate the frequency, clinical and paraclinical features of hepatopulmonary syndrome (HPS) and to determine their predictive values in diagnosis of this syndrome in patients in Iran., Methods: Fifty four cirrhotic patients underwent contrast enhanced echocardiography to detect intrapulmonary and intracardiac shunts by two cardiologists. Arterial blood oxygen, O(2) gradient (A-a) and orthodoxy were measured by arterial blood gas (ABG) test. The patients positive for diagnostic criteria of HPS were defined as clinical HPS cases and those manifesting the intrapulmonary arterial dilation but no other criteria (arterial blood hypoxemia) were defined as lHPS cases. HPS frequency, sensitivity, positive and negative predictive values of clinical and paraclinical features were studied., Results: Ten (18.5%) and seven (13%) cases had clinical and subclinical HPS, respectively. The most common etiology was hepatitis B. Dyspnea (100%) and cyanosis (90%) were the most prevalent clinical features. Dyspnea and clubbing were the most sensitive and specific clinical features respectively. No significant relationship was found between HPS and splenomegaly, ascites, edema, jaundice, oliguria, and collateral veins. HPS was more prevalent in hepatitis B. PaO(2)< 70 and arterial-alveolar gradient had the highest sensitivity in HPS patients. Orthodoxy specificity was 100%., Conclusion: Clubbing with positive predictive value (PPV) of 75% and dyspnea with negative predictive value (NPV) of 75% are the best clinical factors in diagnosis of HPS syndrome. PaO(2)< 70 and P (A-a) O(2)> 30 and their sum, are the most valuable negative and positive predictive values in HPS patients.

The human metapneumovirus (hMPV) was recently identified and linked to acute respiratory tract infections (ARTI). To assess the clinical importance of this virus in infants and children, we developed a rapid and efficient reverse transcription-PCR-based screening method for a large volume of samples and tested retrospectively a collection of 1,132 respiratory specimens submitted over a full year period to the virology laboratory of a large tertiary care pediatric center in Montreal, Canada. A total of 41 samples from 37 patients were positive by this method. During the winter months of 2001, up to 8% of specimens submitted for respiratory virus testing were hMPV positive. Sequencing data of the hMPV M gene revealed that two genogroups of the virus, each of which can be divided into two subgroups, cocirculated during this time period. A case-controlled study was conducted to compare the symptoms associated with hMPV infection with those involving other etiologic agents causing ARTI. Symptoms most frequently observed in hMPV-positive patients were cough, wheezing, and dyspnea, although the symptomatology could differ substantially from patient to patient. No distinct symptom profile could be associated with hMPV. Three nosocomial cases of hMPV infection were identified. Together, our data suggest that hMPV is a significant cause of symptomatic respiratory tract infections in infants and children. The incidence of the disease and the morbidity associated with the infection justify adding hMPV to the list of common respiratory viruses routinely screened for by clinical laboratories.

Aims: To examine the value of the anteroposterior pericardial sac diameter (APD) for prediction of the volume of pericardial effusion., Methods and Results: We measured the APD by echocardiography before 52 pericardiocentesis procedures and correlated it with the aspirate volume, etiology, symptoms, and clinical outcome. The volume of the aspirate ranged from 60 to 2300 ml (median 650 ml). The APD (range 8.0 cm-15.9 cm, median 12 cm) correlated well with the cubic root of the volume of the effusion [volume=(0.8APD-0.6)3, r2=0.533, p<0.01]. An APD>or=12 cm had a positive predictive value of 88% and a negative predictive value of 83% for effusion volume above the sample median (>or=650 ml) and a positive predictive value of 100% for effusion in the middle or upper aspirate volume tertiles. Effort dyspnea was more common among patients with APD>or=12.0 cm (n=13) than in those with APD<12.0 cm (n=11) (p=0.007). One-year survival after pericardiocentesis was closely related to the severity of the underlying etiology and was not influenced by the volume of the effusion before aspiration., Conclusions: The APD is a simple, valuable method for non-invasive prediction of pericardial fluid volume. A greater APD is associated with, and may explain, effort dyspnea.

Background: In order to better define the clinical characterization of pulmonary embolism (PE) in the elderly, the clinical and laboratory findings were compared in older (> or = 65 years old) and younger (< 65 years old) patients., Methods and Results: The study group comprised 149 patients (58 older and 91 younger) who received a final diagnosis of PE and were retrospectively evaluated. The severity of PE was assessed by calculating the pulmonary vascular obstruction scores (PVOs) scintigraphically: PVOs > or = 50% was defined as severe disease. Dyspnea was the most frequent symptom in both groups. Chest pain and hemoptysis were less frequent in older patients (48.3% vs 79.1%, p = 0.001; 6.9% vs 20.9%, p = 0.021, respectively) whereas syncope occurred more often in the older group (27.6% vs 9.9%, p = 0.005). PVOs > or = 50% occurred in 55.1% of older and 32.9% of younger patients (odds ratio: 1.67, 95%confidence interval: 1.118-2.507, p = 0.013)., Conclusions: The clinical presentation of PE can be subtle or atypical in elderly patients and hence they may have more severe disease. Therefore, a high clinical suspicion is required in order to prevent delays in diagnostic work-up and initiation of appropriate treatment.

Objective: To assess the mechanisms through which an enlarged aortic root may facilitate right to left shunting through a patent foramen ovale., Patients: 19 patients with the platypnoea-orthodeoxia syndrome (POS) were compared with 30 control patients without platypnoea., Interventions: Multiplane transoesophageal echocardiography., Main Outcome Measures: The aortic root diameter, atrial septal dimension behind the aortic root, and amplitude of the phasic oscillation of the septum were measured. Four groups of patients were compared: 12 platypnoeic patients with a dilated aortic root (POS-D), 7 platypnoeic patients with a normal aortic root (POS-N), 15 control patients with a dilated aortic root (CONT-D), and 15 control patients with a normal aortic root (CONT-N)., Results: In POS-D and CONT-D patients, the apparent atrial septal dimension was 16.3 (2.7) mm and 17.4 (5.9) mm respectively, compared with 24.4 (5.2) mm in POS-N patients and 25 (4) mm in CONT-N (p < 0.005). Furthermore, the amplitude of septal oscillation was 14.7 (2.5) mm in the POS-D group versus 5.8 (2.4) mm in CONT-N (p < 0.001) compared with 23.3 (3) mm in seven patients with an atrial septal aneurysm (p < 0.001)., Conclusion: Patients with an enlarged aorta have an apparently smaller dimension and increased mobility of the atrial septum. These findings appear to result from compression by the aortic root and decreased septal tautness. Consequently, a "spinnaker effect" with the inferior vena caval flow may take place, opening the foramen ovale and leading to sustained right to left shunting.

Background: Several studies have implicated a role of inflammation in the pathogenesis of lung damage in idiopathic pulmonary fibrosis (IPF). Parenchymal lung damage leads to defects in mechanics and gas exchange and clinically manifests with exertional dyspnea. Investigations of inflammatory cells in IPF have shown that eosinophils, neutrophils and CD8+ TLs may be associated with worse prognosis. We wished to investigate by quantitative immunohistochemistry infiltrating macrophages, neutrophils and T lymphocytes (TLs) subpopulations (CD3+, CD4+ and CD8+) in lung tissue of patients with IPF and their correlation with lung function indices and grade of dyspnoea., Methods: Surgical biopsies of 12 patients with IPF were immunohistochemically stained with mouse monoclonal antibodies (anti-CD68 for macrophages, anti-elastase for neutrophils, and anti-CD3, anti-CD4, anti-CD8 for CD3+TLs, CD4+TLs, and CD8+TLs respectively). The number of positively stained cells was determined by observer-interactive computerized image analysis (SAMBA microscopic image processor). Cell numbers were expressed in percentage of immunopositive nuclear surface in relation to the total nuclear surface of infiltrative cells within the tissue (labeling Index). Correlations were performed between cell numbers and physiological indices [FEV1, FVC, TLC, DLCO, PaO2, PaCO2 and P(A-a)O2)] as well as dyspnoea scores assessed by the Medical Research Council (MRC) scale., Results: Elastase positive cells accounted for the 7.04% +/- 1.1 of total cells, CD68+ cells for the 16.6% +/- 2, CD3+ TLs for the 28.8% +/- 7, CD4+ TLs for the 14.5 +/- 4 and CD8+ TLs for the 13.8 +/- 4. CD8+TLs correlated inversely with FVC % predicted (rs = -0.67, p = 0.01), TLC % predicted (rs = -0.68, p = 0.01), DLCO % predicted (rs = -0.61, p = 0.04), and PaO2 (rs = -0.60, p = 0.04). Positive correlations were found between CD8+TLs and P(A-a)O2 (rs = 0.65, p = 0.02) and CD8+TLs and MRC score (rs = 0.63, p = 0.02). Additionally, CD68+ cells presented negative correlations with both FVC % predicted (rs = -0.80, p = 0.002) and FEV1 % predicted (rs = -0.68, p = 0.01)., Conclusion: In UIP/IPF tissue infiltrating mononuclear cells and especially CD8+ TLs are associated with the grade of dyspnoea and functional parameters of disease severity implicating that they might play a role in its pathogenesis.

Swyer-James (MacLeod) syndrome is an acquired form of unilateral hyperlucency of the lung and is characterized by the development of severe emphysema, bronchiectasis, and/or bronchiolitis obliterans. It may develop as a complication of repeated episodes of pulmonary infection resulting in bronchiolitis obliterans and obstruction of small airways. Most patients with Swyer-James (MacLeod) syndrome can be managed clinically, and the pathologic features of the syndrome have been described in only a few reports. Placental transmogrification of the lung is a rare histopathologic finding that has been described in patients with severe emphysema associated with cigarette smoking, congenital bullous emphysema, and fibrochondromatous hamartomas of the lung and is characterized by the development of peculiar structures in the pulmonary parenchyma that resemble placental villi. To our knowledge, placental transmogrification of the lung has not been previously described in patients with Swyer-James (MacLeod) syndrome. We encountered a 32-year-old man with a history of childhood asthma who presented with progressively severe exertional dyspnea and had unilateral right lung hyperlucency. The patient underwent a right pneumonectomy. Examination of the lung revealed severe mixed centriacinar-panacinar emphysema in all lobes, bullous emphysema in the upper lobe, bronchiectases, mild interstitial pneumonia with fibrosis, and placental transmogrification of the pulmonary parenchyma of all 3 lobes. Here, we review the pathology of Swyer-James (MacLeod) syndrome and the possible pathogenesis of villous-like changes in the lung tissues.

This study aims to investigate the possibility of additional value of leukotriene receptor antagonist (LTA) on dyspnea score, arterial blood gases (ABG), pulmonary function tests (PFTs), and quality of life (St. George QoL) in chronic obstructive pulmonary disease (COPD) patients. In this randomized, prospective, single-blind, and controlled study, 117 non-reversible COPD patients defined by global initiative for chronic obstructive lung disease (GOLD) criteria were randomized to receive ipratropium bromide, formoterol and montelukast (n:58, montelukast group) or ipratropium bromide and formoterol (n:59, control group) after a 2-week run-in period. There was no significant demographic difference between the two groups (P>0.05). Baseline ABG, PFT, visual analoque scores (VAS), and QoL scores were obtained and at first month and second month, PFT, VAS, and QoL scores were repeated and ABG was obtained at second month and the values were compared with baseline values. As the result of the comparison, there was significant increase in vital capacity, FVC, FEV1, VAS, and PaO2 parameters (P<0.05), and a significant decrease in the QoL scores (P<0.05) in the montelukast group. These parameters did not show any difference in the control group (P>0.05). Sputum samples that could be obtained in 24 of the COPD patients were evaluated and in the montelukast group, there was a decrease in neutrophilic activity after treatment (n:13) (P:0.059). These results suggest that LTA that is used additionally in routine treatment protocol can produce additive improvement on PFT, dyspnea score and especially QoL in patients with stable COPD and for these reasons, LTA may be taken into account when there is need for an additional anti-inflammatory treatment in COPD patients.

The objective of this study was to evaluate the risk factors associated with mortality in interstitial lung disease patients. We performed a retrospective study of 722 consecutive patients submitted for lung biopsy during the 1986-1990 period. Twenty-two (3%) died within the 30 days following surgery. Forty-four patients who survived after the surgery for the same time span were randomly chosen as control group. Dyspnea at rest was present in 18/44 of surviving group (SG) and in 18/22 of the nonsurviving group (NSG) (OR 6.5, 95% CI 1.8-22.4,p = .001). Systemic diseases (i.e., diabetes, systemic arterial hypertension)were mainly present in the NSG (OR 7.2, 95% CI 2.3-22.8, p < .001). The SG displayed significantly less respiratory insufficiency with a PaO2 of 52.2 + 8.4 versus 38.5 i 9.4 mm Hg, and PaCO2 of 28.8 i 4.5 versus 38.5 +/- 9.2 mm Hg, respectively (p < .001). Likewise, the SG exhibited a PaCO2/PaO2 ratio of 0.5 - 0.1, while in the NSG it was of 1 +/- 0.4 (p < .001), showing a sensitivity of 84% and specificity of 93% for mortality. Multiple logistic regression analysis for these variables showed that log likelihood was still significant for PaCO2 > 34 mm Hg, PaO2 <48 mm Hg, and comorbid diseases. Logistic regression analysis of these three variables showed the greatest sensitivity and specificity (84 and 750/0,respectively) for prediction of mortality. However, the strongest association was found when PaCO2/PaO2 ratio was analyzed alone (OR 21,073,CI 95% 28-15,946,357, p < .005). These data suggest that PaCO2/PaO2 ratio appears to be a predictor of mortality in this subset of patients. Its prospective use has reduced early mortality after surgery less than 1% in the last decade.

Pulmonary hamartoma usually occurs as a benign, well-circumscribed single nodule in the lung parenchyma. We report on a unique case of hamartoma that extended along the bronchial tree, formed endobronchial polypoid lesions, and expanded into the lung parenchyma. The patient, a 48-year-old man, was admitted with dyspnea and chest pain. A transbronchial biopsy was performed on a tumorous lesion that was diagnosed histologically as a hamartoma. As this lesion was found to be growing diffusely along the bronchial tree, a left pneumonectomy was performed. Gross examination showed that yellowish soft tissue had surrounded the bronchial tree and extended into the lung parenchyma. Histologically, the lesion contained mainly mature adipose tissue, cartilage, and muscle tissue with a minor component of short spindle cells in a myxomatous matrix. The patient was diagnosed as having diffuse pulmonary hamartoma.

The aim of this study was to evaluate the safety and efficacy of azathioprine in the treatment of interstitial lung disease (ILD) associated with systemic sclerosis (SSc). The records of patients with SSc with ILD who were treated with azathioprine were reviewed. Patients were treated with azathioprine and low-dose prednisone if they had progressive pulmonary symptoms (deterioration in the dyspnea score) or poor or deteriorating lung function. Response was classified as improved if the FVC increased more than 10% from baseline, and stable if it remained within 10% of baseline. Serial dyspnea scores were recorded. Eleven patients were treated with azathioprine, three of whom received treatment for 6 months or less owing to adverse effects (nausea, leukopenia and pulmonary tuberculosis in one patient each). The remaining eight patients received at least 12 months' treatment and the results suggested an improvement in the mean percent predicted FVC from a baseline value of 54.25+/-3.53 to 63.38+/-6.15 after 12 months ( p=0.101). Overall, five patients improved and three remained stable. The mean dyspnea score ( n=8) improved from a baseline of 1.55+/-0.19 to 0.50+/-0.19 at 12 months ( p=0.011). This is the first case series of patients with SSc-associated ILD treated with azathioprine. Our results suggest that azathioprine may have a role in stabilizing lung function and improving symptoms in SSc, although this needs confirmation by a randomized controlled trial.

Objective: Medically unexplained dyspnea is common in adult and accounts for 14% patients complaining of dyspnea. Its occurrence in children is seldom recognized. In the present paper, 34 children with medically unexplained dyspnea (age 10 to 18 years) seen in Peking Union Medical College Hospital from 1996 to 2002 are reported., Methods: The diagnosis of medically unexplained dyspnea was clinical: it was based on the presence of dyspnea and other complaints which cannot be explained by an organic disease. The patients answered Nijmegen questionnaire and state and trait anxiety (STAI), and performed hyperventilation provocation test. Twenty sessions of breathing therapy were applied and 13 out of 34 children were followed up after the therapy., Results: Among the children, 75% started to have symptoms at the age of 13 to 16 years, though the age of first episode could be as early as 8 years. In most of the cases, the course was chronic clinically. In addition to marked dyspnea, their clinical profile included symptoms of hyperventilation i.e. blurred vision, dizziness, tingling, stiff fingers or arm. The symptoms of anxiety were less frequent in children and accordingly the level of anxiety evaluated by means of STAI was lower in children compared to adult patients. The precipitating psychological factors appeared to be related to middle school competition. Pressure from exams, reprimand from stern and unsympathetic teachers coupled with high parental expectation could be emotionally damaging to psychologically susceptible children. Thirteen patients were followed up after 2-3 months of breathing therapy with emphasis on abdominal breathing and slowing down of expiration. After therapy, the sum score of the Nijmegen Questionnaire was markedly decreased. Dyspnea and symptoms of hyperventilation were improved. The level of anxiety was minimally modified., Conclusion: The cases illustrated the need for careful diagnostic evaluation and treatment because of the high rate of chronicity of the disorder.

The patient is a 62-year-old man who was diagnosed with myasthenia gravis and invasive thymoma at the age of 45 years, and had received treatment by extended thymectomy and radiotherapy. At the age of 61, he had suffered from a myasthenic crisis, and been administered immunoadsorption therapy under managed ventilatory care. Treatment had then been continued with steroids; however, due to subsequent deterioration of his diabetic state, treatment was switched to the immunosuppressant drug tacrolimus. Three months after the commencement of tacrolimus administration, the patient developed generalized malaise and dyspnea. The serum creatine phosphokinase (CPK) level was abnormally elevated, and abnormal electrocardiographic findings were noted, including atrioventricular dissociation and ventricular escape contraction. Steroid pulse therapy was therefore initiated, however, 4 days later, the patient suddenly died. Autopsy examination revealed inflammatory cell infiltration with giant cells in the myocardium, diffuse myocardial degeneration, and polymyositis. The case was therefore considered as one with the syndrome of myasthenia gravis, polymyositis, giant cell myocarditis, and thymoma.

Background: This is the first comparison of two combination therapies, fluticasone propionate/salmeterol and ipratropium bromide/albuterol (salbutamol), for the treatment of patients with COPD., Methods: A randomized, double-blind, double-dummy, parallel group, multicenter evaluation of fluticasone propionate/salmeterol 250/50 microg twice daily via DISKUS and ipratropium bromide/albuterol 36/206 microg four times daily via metered-dose inhaler over 8 weeks was conducted at 41 research sites in the US. Morning pre-dose FEV(1), 6-hour serial spirometry, PEF, dyspnea, night-time awakenings, supplemental albuterol use, and patient diary evaluations of symptoms were evaluated., Results: A total of 365 patients with symptomatic COPD were enrolled. The treatment groups were similar in mean age (63.3 and 63.9 years), screening pulmonary function (44.1% and 43.2% of predicted FEV(1)), race (96% and 95% White), and sex distribution (59% and 60% male). Both fluticasone propionate/salmeterol and ipratropium bromide/albuterol improved lung function, symptoms, and supplemental albuterol use compared with baseline. Fluticasone propionate/salmeterol was more effective than ipratropium bromide/albuterol for improvement in morning pre-dose FEV(1), morning PEF, 6-hour FEV(1) area under the curve (AUC(6)), Transition Dyspnea Index (TDI) focal score, daytime symptom score, night-time awakenings, sleep symptoms, and albuterol-free nights (p < or = 0.013). Compared with day 1, at week 8 the FEV(1) AUC(6) significantly increased with fluticasone propionate/salmeterol and significantly decreased with ipratropium bromide/albuterol (p < or = 0.003). The incidence of adverse events was similar between treatment groups, except for a higher incidence of oral candidiasis with fluticasone propionate/salmeterol., Conclusions: Short-term treatment with the combined inhaled corticosteroid and long-acting beta(2)-adrenoceptor agonist fluticasone propionate/salmeterol resulted in greater control of lung function and symptoms than combined ipratropium bromide/albuterol bronchodilator therapy, in patients with COPD.

Fever of unknown origin (FUO) is a common syndrome. A total of 94 patients (57 men and 37 women; mean age, 56.3 +/- 19 years, range, 18-86 years) who met the criteria of FUO were included in this study. Mycobacteriosis was diagnosed in 22 (23%) of these patients (13 men and 9 women), including 9 with disseminated disease and 13 with pulmonary disease. There was no significant statistical difference in age, sex, short-term survival status (3 months), and other clinical parameters between patients with and without mycobacteriosis. Clinical manifestations may be specific or nonspecific. The most common initial presentations in patients with mycobacteriosis were respiratory tract symptoms, mainly of cough and dyspnea, observed in 11 (50%) patients, and disturbance of consciousness in 6 (27%). The associated conditions included malnutrition (4 patients, 18%), diabetes mellitus (3, 14%), and renal failure (3, 14%). Four (18%) patients had a history of pulmonary tuberculosis or tuberculous spondylitis in their early adulthood. The 2 most common findings on chest radiograph were interstitial (41%) and nonspecific infiltrative (32%) patterns. In conclusion, mycobacteriosis remains the leading cause of FUO in southern Taiwan and it is important to screen for this treatable disease in all cases of FUO.

Background: Cytomegalovirus (CMV) infection and Kaposi's sarcoma (KS) are common in AIDS patients but rarely involve the thyroid, and coexistence of these two entities in that organ has not yet been described before., Case: A 41-year-old female AIDS patient presented with a 2 x 1-cm, well-demarcated, rubbery mass in the right side of the thyroid. On fine needle aspiration (FNA), spindle cells were retrieved singly or in small, loose clusters; they had bland, fusiform to cigar-shaped nuclei; inconspicuous nucleoli; delicate cytoplasmic vacuoles; cytoplasmic hyaline drops; and hemosiderin granules. A single endothelial cell showed an enlarged nucleus with a basophilic intranuclear inclusion and periinclusional halo., Conclusion: This is the first reported case of an AIDS patient with KS and CMV infection simultaneously involving the thyroid diagnosed by FNA.

Background: Dyspnea is the major limiting symptom for patients with chronic obstructive pulmonary disease (COPD). This study was designed to investigate the impact of the combination of two bronchodilators on perception of dyspnea in these patients., Methods: Twenty patients with moderate to severe COPD (age was [mean +/- standard deviation (SD)] 64 +/- 7 years, FEV1/FVC ratio was 45 +/- 11%) were randomized in a crossover, double-blind, placebo-controlled clinical trial to receive two puffs of either ipratropium bromide plus salbutamol (IB+S), IB+placebo (P), S+P, or P+P through metered dose inhalers (MDIs) daily over a 2-week period for each combination. At the end of each period, spirometry was obtained at baseline and 15, 30, 60, and 120 min after bronchodilator. Dyspnea was evaluated using a Borg scale during a 12-min walking test (12-MWT) performed at baseline and 120 min after MDIs use. In addition, the dyspnea component of the Chronic Respiratory Disease Questionnaire (CRDQ) and a diary of symptoms were used to evaluate this symptom., Results: After 15 days of treatment, FEV1 improved significantly with all three combinations of MDIs containing bronchodilator agents. In comparison with placebo, significant improvement in dyspnea during 12-MWT was observed only when the combination of IB+S was used. No significant changes in CRDQ or patients' symptom diaries were observed with any combinations used., Conclusions: Combination of the two bronchodilators used in this study appears to alleviate perception of dyspnea more than use of either agent alone.

Background: Increased pulmonary capillary pressure (PAWP) and decreased serum colloid osmotic pressure (COP) are important factors in pulmonary edema (PE) formation. The aim of this study was to evaluate the clinical usefulness of noninvasive determination of COP, PAWP and COP-PAWP gradient in patients with acute dyspnea., Methods: Thirty four patients presenting with PE, 19 with preserved left ventricular systolic function (Ia) and 15 with depressed systolic function (Ib), and 26 patients presenting with acute pulmonary diseases (II) were enrolled. COP was estimated using Landis and Pappenheimer formula (COP = 2.1 x P + 0.16 x P2 + 0.009 x P3, P: serum total protein concentration). PAWP was measured by transthoracic Doppler echocardiography using E/Vp and 1000/(2 x IPT + Vp) Doppler indexes., Results: The mean value of the gradient was 0.5 +/- 5, 1.7 +/- 3.4 and 10.4 +/- 4.7 mmHg in Ia, Ib and II respectively (P < 0.001 Ia and Ib vs II). PAWP was higher in Ia and Ib than in II (P < 0.001). However, hypoalbuminemia with COP < or = 18 mmHg was observed in 63% of patients in Ia as compared to those in Ib (7%) and II (8%)., Conclusions: Severe hypoalbuminemia frequently contributes to PE formation in the presence of normal left ventricular systolic function. A COP-PAWP gradient value lower than 6 mmHg is highly predictive of the diagnosis of acute heart failure in these patients presenting with acute dyspnea.

Eleven subjects, aged between 15 and 60 years, presenting with diffuse infiltrative lung disease (DILD) and progressive dyspnoea, underwent an open lung biopsy (OLB). The authors feel that OLB does give a confidence to the treating physician to begin with a specific therapy in the form of steroids. But, as a matter of fact, at most health care delivery centres in the country, facilities for OLB are not available. Hence, the specific therapy should be instituted presumptively following an overall suggestion of disease based upon the clinical, physiological (chiefly comprising the pulmonary function test or PFT) and the radiological criteria, so that progression of disease could be arrested at an early stage.

A 41-year-old man complained of subacute onset of dyspnea and pain in the neck and chest. He was diagnosed with bilateral diaphragmatic paralysis, based on clinical inspection of the breathing pattern and transdiaphragmatic pressure recording, and was trained to use a portable bi-level positive airway pressure apparatus (BiPAP). Needle electromyography showed profuse fibrillation potentials and positive waves in the diaphragm, more abundant on the right than left side, and no response to phrenic nerve stimulation. Other muscles were not involved. Follow-up examinations, performed at 9 and 12 months after onset of paralysis, demonstrated a slow but progressive improvement of the patient's respiratory function, together with the appearance of reinnervation potentials in the diaphragm, and polyphasic, long-latency responses to phrenic nerve stimulation. The subacute onset of the paralysis associated with local pain, and its subsequent recovery, suggest bilateral proximal lesions in the phrenic nerves. In the absence of traumatic or metabolic causes, these findings suggest that the phrenic nerve can be a target in idiopathic neuritis., (Copyright 2002 Wiley Periodicals, Inc.)

To our knowledge, no data have been provided as to whether and to what extent dynamic hyperinflation, through its deleterious effect on inspiratory muscle function, affects the perception of dyspnoea during induced bronchoconstriction in patients with chronic airflow obstruction. We hypothesized that dynamic hyperinflation accounts in part for the variability in dyspnoea during acute bronchoconstriction. We therefore studied 39 consecutive clinically stable patients whose pulmonary function data were as follows (% of predicted value): vital capacity (VC), 97.8% (S.D. 16.0%); functional residual capacity, 105.0% (18.8%); actual forced expiratory volume in 1 s (FEV(1))/VC ratio, 56.1% (6.3%). Perception of dyspnoea using the Borg scale was assessed during a methacholine-induced fall in FEV(1). The clinical score and the treatment score, the level of bronchial hyper-responsiveness and the cytological sputum differential count were also assessed. In each patient, the percentage fall in FEV(1) and the concurrent Borg rating were linearly related, with the mean slope (PD slope) being 0.09 (0.06). The percentage fall in FEV(1) accounted for between 41% and 94% of the variation in the Borg score. At a 20% fall in FEV(1), the decrease in inspiratory capacity (Delta IC) was 0.156 (0.050) litres. Patients were divided into three subgroups according to the PD slope (arbitrary units/% fall in FEV(1)): subgroup I [eight hypoperceivers; PD slope 0.026 (0.005)], subgroup II [26 moderate perceivers; 0.090 (0.037)] and subgroup III [five hyperperceivers; 0.200 (0.044)]. By applying stepwise multiple regression analysis with the PD slope as the dependent variable, and other characteristics (demographic, clinical and functional characteristics, smoking history, level of bronchial hyper-responsiveness and sputum cytological profile) as independent variables, Delta IC (r(2)=45%, P<0.00001) and to a lesser extent treatment score (r(2)=17.3%, P<0.0006), and to an even lesser extent age (r(2)=3%, P<0.05), independently predicted a substantial amount (r(2)=65.27%, P<0.00001) of the variability in the Borg slope. Thus acute hyperinflation, and to a lesser extent treatment score and age, account in part for the variability in the perception of dyspnoea after accounting for changes in FEV(1) during bronchoconstriction in patients with chronic airflow obstruction.

Post-pneumonectomy syndrome is an unusual condition, that can occur a variable period of time after a patient has had a pneumonectomy. Management of this syndrome has been described using a number of different techniques, often with considerable mortality. We present a case report where this condition was treated successfully by insertion of an expandable Nitinol stent. This is the first time this technique has been described to treat this condition, and we feel it may be the procedure of choice in managing these patients.